Androgen insensitivity syndrome (AIS) is a disorder of sexual development characterized by a female phenotype with a 46 XY karyotype. Most patients present with primary amenorrhea; however,1.1% of patients present with an inguinal mass. Most commonly, seminoma arising from the gonads are found.                                                                                This report represents the case of a 15 year-old female looking adolescent who initially presented with an abdominopelvic mass. A diagnosis of AIS was made based on the physical examination findings, endocrine profiling, imaging studies and karyotyping. She underwent cytoscopy, exploratory laparotomy,adhesiolysis, tumor debulking, frozen section, bowel run, repair of serosal tear, Jackson-Pratt drain insertion, bilateral percutaneous nephrostomy under combined spinal and epidural anesthesia. Histopathologic examination of the excised mass revealed a mixed germ cell tumor. This paper will discuss the diagnostic approach as well as the management and prognosis of patients with AIS associated with mixed germ cell tumor.

Human ; Female ; Adolescent ; Androgen-insensitivity Syndrome ; Gonadoblastoma ; Feminization ; Germ Cells ; Feminization ; Adolescent ; Testosterone ; Genitalia ; Phenotype ; Karyotype

After infection of male mice with the plerocercoids (spargana) of Spirometra mansoni, serum levels of estrogen and testicular weight were analyzed by enzyme-linked immunosorbent assay (ELISA) and weighing machine, respectively. The serum level of estrogen increased progressively in infected mice compared with normal controls, whereas the testicular weight of infected mice decreased significantly (P < 0.05). These results suggest that certain substances from spargana change the steroid hormone metabolisms in the host by unknown pathways, and chronic infection may contribute to change of the function of steroid hormone target organ, i.e., testis, in male mice.
Time Factors ; Testis/*parasitology/pathology ; Sparganosis/*complications ; Organ Size ; Mice ; Male ; Feminization/*parasitology ; Estrogens/blood ; Animals

Adrenal cortical carcinoma is a rare disease both in adults and in children. Most of these tumors are functional, especially in children, producing endocrine syndromes such as virilization, Cushing syndrome, hyperaldosteronism or feminization. We experienced a case of adrenal cortical carcinoma in a 7yr old boy who showed features of virilization such as rapid growth rate, penile enlargement, and pubic hair. This case was diagnosed with typical hormonal findings and abdominal MRI and confirmed by pathologic findings. He was successfully treated by total left adrenalectomy and has been followed up without problem over eight months.
Adrenalectomy ; Adrenocortical Carcinoma* ; Adult ; Child ; Cushing Syndrome ; Feminization ; Hair ; Humans ; Hyperaldosteronism ; Magnetic Resonance Imaging ; Male ; Rare Diseases ; Virilism

This is the first report of motor neuron disease associated with feminization in Korea. The case in this report is a 26 year old male patient wlth hypogonadotropic hypogonadism who developed progressive lower motor neuron dysfunctions started from the lower extremities and later spreaded to the entire body including bulbar muscles. At first glance, wve thought that this is very similar to well known Kennedy-Alter-Sung syndrome but after thorough evaluation and follow up of the partient for 4 years until his death we concluded that this case is somewhat different from Kennedy-Alter-Sung syndrome in several aspects even though it is similar and may be a variant of that syndrome or a new unique syndrome associated with hypogonadotropic hypogonadism.
Adult ; Feminization ; Follow-Up Studies ; Humans ; Hypogonadism ; Korea ; Lower Extremity ; Male ; Motor Neuron Disease* ; Motor Neurons* ; Muscles

Adrenal cortical tumor is a rare disease both in adults and children. Most of these tumors are malignant and functional, especially in children, producing endocrine syndrome such as virilization, Cushing's syndrome, hyperaldosteronism or feminization. Recently, we experienced a case of adrenal cortical tumor in a 26 month-old boy who showed features of virilization. This case was diagnosed with typical hormonal findings and abdominal CT and confirmed by pathologic examinations. He was successfully treated with right adrenalectomy. We report a case of virilizing adrenal cortical tumor and review the literature.
Adrenalectomy ; Adult ; Child* ; Child, Preschool ; Cushing Syndrome ; Feminization ; Humans ; Hyperaldosteronism ; Male ; Rare Diseases ; Tomography, X-Ray Computed ; Virilism

Testicular feminization is an uncommon genetic disorder with considerably familial predisposition and results in total feminization due to end-organ unresponsiveness to androgens. It is characterized by the presence of testes in phenotypically female with adequate breast development, normal extemal genitalia, absence of mullerian structures, and meager or absence of body hair. These patients characteristically have male karyotype(XY) and negative sex chromatin and are at increased risk of undergoing malignant transformation of the undescended gonad. In recent times, the malignant potential of the dysgenetic gonads in the intersex patients with a Y chromosome has been stressed by many authors, but few reports of an association between testicular feminization syndrome and benign tumors such as Sertoli cell adenomas. In the present study, postoperative pathology revealed that the gonads were Sertoli cell adenomas. The main features of clinical presentation and histological studies are briefly discussed with a review of the literature.
Adenoma* ; Androgen-Insensitivity Syndrome* ; Androgens ; Breast ; Female ; Feminization ; Genitalia ; Gonads ; Hair ; Humans ; Male ; Pathology ; Sex Chromatin ; Testis ; Y Chromosome

Adrenocortical oncocytoma is a rare adrenal gland tumor, and in most cases, benign and non-functioning. Functional adrenocortical oncocytoma has only been reported in eleven cases worldwide, including three children. These cases all showed clinical symptoms and signs, such as virilization, feminization, Cushing syndrome and Conn syndrome, as a result of overproduction of adrenal steroid hormones. We report a 2-year-old girl who presented with precocious puberty with premature pubarche. Dehydroepiandrosterone sulfate (DHEA-S) and testosterone levels were elevated and a 1.9 cm right adrenocortical oncocytoma was found. After tumor resection, her hormone levels were normalized.
Adenoma, Oxyphilic ; Adrenal Glands ; Child ; Cushing Syndrome ; Dehydroepiandrosterone Sulfate ; Feminization ; Humans ; Hyperaldosteronism ; Preschool Child ; Puberty, Precocious ; Testosterone ; Virilism

Transsexual individuals with gender dysphoria or gender identity disorder are rare, with a prevalence reported to range from 0.002% to 0.014%. Studies have shown that mastectomy yields significant improvements in body image and self-esteem in female-to-male transsexual patients. In patients with grade III breast ptosis, mastectomy with a nipple-areolar complex (NAC) graft is the most commonly used technique, although it has several disadvantages. In the case described herein, a bilateral mastectomy preserving the NAC in an inferior pedicle was performed. Additionally, a thin superior thoracic dermal-fat flap was preserved and eventually sutured at the previous inframammary fold, preventing an inverted T scar. This case shows the advantage of this technique for preserving the blood supply and innervation of the NAC, with a low hypopigmentation risk. Furthermore, in this technique, the patch effect does not impair the results of the NAC graft, and there is no need to use an inverted T scar that may result in thoracic feminization.
Body Image ; Breast ; Cicatrix ; Feminization ; Gender Dysphoria ; Gender Identity ; Gynecomastia ; Humans ; Hypopigmentation ; Male ; Mastectomy ; Prevalence ; Thorax ; Transplants ; Transsexualism

BACKGROUND: This study examined the clinical effects of leuprolide acetate in sexual offenders with paraphilic disorders evaluated by means of objective psychiatric assessment. METHODS: The subjects of this study were seven sexual offenders who were being treated by means of an injection for sexual impulse control by a court order. They had been diagnosed with paraphilia by a psychiatrist based on the Diagnostic and Statistical Manual of Mental Disorders, 5th edition (DSM-5) and had been put on probation by the Ministry of Justice between January 2016 and December 2016. RESULTS: After twelve months, we observed significant improvement in symptoms, as decrease of abnormal sexual interest and activity, sexual fantasy, Clinical Global Impression-Severity (CGI-S), and Clinical Global Impression-Impulsivity (GCI-I). There were a mild feminization of the body shape, feelings of fatigue, and mild hot flushes. No other adverse effect was reported. CONCLUSION: These results suggested that the clinical effects of leuprolide acetate in sexual offenders might be an effective treatment and safety strategy.
Criminals* ; Diagnostic and Statistical Manual of Mental Disorders ; Fantasy ; Fatigue ; Feminization ; Humans ; Leuprolide* ; Paraphilic Disorders* ; Psychiatry ; Sexual Behavior ; Social Justice ; Therapeutic Uses*

Adrenocortical carcinoma is a rare malignant endocrine tumor which accounts for about 0.05% to 0.2% of all carcinomas. Three clinical patterns can be encountered. In 30% of the cases, a mass syndrome without any clinical evidence of hypersecretion is presented as abdominal pain, a palpable abdominal mass, abdominal distension, and weight loss. In 60% of the cases, an overt clinical syndrome of hypersecretion is of almost purely hypercortisolism in 30% of such cases, vilirization in 22%, feminization in 10%, hyperaldosteronism in 2.5%, and a mixed secretion in 35%. In the remaining 10% of the cases, an adrenal `incidentaloma' is found incidentally during evaluation of the other disease. About 70% to 80% of patients are diagnosed with an advanced stage (III or IV) and metastasis has occured in 20% to 40% of the patients at the time of presentation. Early surgery an with adrenalectomy is the only means of cure. The prognosis is poor with a 5-years survival rate of 16% to 34% due to initial diagnosis at an advanced stage. Recently, we experienced two cases of adrenocortical carcinomas. In case I, a 62-year-old female patient presented with a clinical syndrome of hypercortisolism and had an extensive local invasion of stage IV; in case II, a 49-year-old male patient complained of abdominal pain and distension without any clinical syndrome of hypersecretion and had stage IV liver metastasis.
Abdominal Pain ; Adrenalectomy ; Adrenocortical Carcinoma* ; Cushing Syndrome ; Diagnosis ; Female ; Feminization ; Humans ; Hyperaldosteronism ; Liver ; Male ; Middle Aged ; Neoplasm Metastasis ; Prognosis ; Survival Rate ; Weight Loss